Title

Ripor2 is Involved in Auditory Hair Cell Stereociliary Bundle Structure and Orientation

Document Type

Article

Publication Date

11-2018

Publication Title

Journal of Molecular Medicine

Keywords

Deafness, Hair cells, Kinocilium, Myh9, Ripor2, Stereocilia

ISSN

0946-2716

Volume

96

Issue/No.

11

First Page

1227

Last Page

1238

Abstract

RIPOR2 (previously known as FAM65B) localizes to stereocilia of auditory hair cells and causes deafness when its function is disturbed by mutations. Here, we demonstrate that during the morphogenesis of the hair cell bundle, absence of Ripor2 affects the orientation of this key subcellular structure. We show that Ripor2 interacts with Myh9, a protein encoded by a known deafness gene. Absence of Ripor2 is associated with low Myh9 abundance in the mouse cochlea despite increased amount of Myh9 transcripts. While Myh9 is mainly expressed in stereocilia, a phosphorylated form of Myh9 is particularly enriched in the kinocilium. In Ripor2-deficient mice, kinocilium shows an aberrant localization which associates with a reduced content of phosphorylated Myh9. Acetylated alpha tubulin, another specific kinociliary protein which contributes to microtubule stabilization, is reduced in the absence of Ripor2 as well. We propose that Ripor2 deficiency influences abundance and/or post-translational modifications of proteins expressed in both stereocilia and kinocilia. This effect may have a negative impact on the structure and function of the auditory hair cell bundle.

Comments

©Springer-Verlag GmbH Germany, part of Springer Nature 2018

Additional Comments

NIH grant #: R01DC009645

DOI

10.1007/s00109-018-1694-x

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Peer Reviewed

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